隆突性皮肤纤维肉瘤 (DFSP) 是一种皮肤肉瘤，具有浸润性生长模式，使得清除边缘具有挑战性。有关 DFSP 自然史、管理和结果的高质量数据有限。回顾性收集了在 8 个国家的 10 个机构接受切除的成年 DFSP 患者的数据。对人口统计学、肿瘤特征、治疗策略和结果进行了分析。分析包括 347 名患者，其中包括年轻（中位年龄 42 岁）、白人（76.2%）、男性（54.2%），伴有躯干病变（57.3%）。大多数 (76.8%) 就诊时有症状。 55.9%的病例使用了术前影像学检查。 50.9% 的病例通过切除活检确诊，25.0% 的病例通过切开活检确诊。尽管 67.4% 的病例计划切缘 >1.0 cm，但只有 69.0% 的患者实现了 R0 切除。 22% 的患者至少接受了一次再次切除。第二次手术实现 R0 切除率为 80.2%，第三次手术实现 R0 切除率为 86.2%。最终，89.5% 的患者可以进行 R0 切除。 12.6% 观察到纤维肉瘤转化 (FST)。总共 6.6% (N = 23) 复发（17 例本地，6 例远处）。在 6 例远处复发中，50.0% 有 FST。中位随访时间为 47.0 个月，疾病特异性生存率为 98.8%。在多变量分析中，指数切除时的 R0 切缘与较宽的圆周切缘和非 FST 组织学相关。在这个国际多中心协作中，DFSP 实践模式是异质的，但取得了良好的复发率和生存率。多次切除以清除边缘仍然很常见，并且可以为未来优化边缘选择的努力提供信息。© 2024 美国癌症协会。

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with an infiltrative growth pattern that makes it challenging to clear margins. High quality data regarding DFSP natural history, management, and outcomes are limited.Data were retrospectively collected for adult DFSP patients who underwent resection at 10 institutions in eight countries. Demographics, tumor characteristics, treatment strategies, and outcomes were analyzed.Analysis included 347 patients consisting of young (median, 42 years), White (76.2%), males (54.2%) with truncal lesions (57.3%). The majority (76.8%) were symptomatic at presentation. Preoperative imaging was used in 55.9% of cases. Diagnosis was established with excisional biopsy in 50.9% versus incisional biopsy in 25.0% of cases. Despite planned margins of >1.0 cm in 67.4% of cases, only 69.0% of patients achieved R0 resection. Twenty-two percent of patients underwent at least one re-excision. R0 resection was achieved at a second procedure in 80.2% and a third procedure in 86.2%. Ultimately, R0 resection was feasible in 89.5% of all patients. Fibrosarcomatous transformation (FST) was observed in 12.6%. In total, 6.6% (N = 23) recurred (17 local, six distant). Of the six distant recurrences, 50.0% had FST. With a median follow-up of 47.0 months, disease-specific survival rate was 98.8%. In multivariable analysis, R0 margins at index resection were associated with wider circumferential margins and non-FST histology.In this international, multicenter collaborative, DFSP practice patterns were heterogeneous but achieved favorable recurrence rates and survival. Multiple excisions to clear margins remain commonplace and can inform future efforts to optimize margin selection.© 2024 American Cancer Society.